Research to help children with cerebral palsy

New research to improve the
bones and muscles of children with cerebral
palsy

As part of ‘Steptember’, the
fund-raising month for people with cerebral palsy, three
researchers from the Auckland Bioengineering Institute (ABI)
will talk about their potentially life-changing research
into the disease.

Cerebral palsy is a movement
disorder caused by damage to parts of the brain before,
during and after childbirth, that affect a child’s ability
to control movement, balance and posture. It is the leading
cause of childhood disability in New Zealand.

It is not a
progressive neural disease – the lesions that cause the
disease are static – yet paradoxically the musculoskeletal
systems of children worsen over time. Using a combination
of imaging techniques and computational modelling
researchers at ABI hope to better understand why.

That
includes a project led by Dr Geoffrey Handsfield, who was
awarded a $1 million Aotearoa Foundation Fellowship.
“Broadly speaking, the goal is to better understand the
progression of cerebral palsy in the musculoskeletal system,
particularly how the disease worsens over time and impairs
muscles, bones and walking ability," he says.

His team
includes Stephanie Khuu, a physiologist now doing a PhD in
Bioengineering, who is looking at why the bones of children
don't regenerate after exercise.

“Typically, when
children exercise their muscles, the muscle is injured, but
then it regenerates. Our muscles do that on a day by day
basis.” Damaging our muscles can even produce
physiological adaptations that actually improve performance
over time, similar to the improvements you see after
‘damaging’ your muscles in the gym by working out.
“But we think that with CP something is going wrong that
doesn’t allow them to regenerate, and so over time, they
progressively degenerate.”

Understanding why things go
wrong requires understanding why things go right. Which is
why Ms Khuu is investigating the process of muscle
regeneration in both typically developing muscles and in the
muscles of individuals with cerebral palsy.

Building a
computational model of the environment in which muscles
regenerate in normal circumstances will help clarify what
hampers those regeneration pathways in children with
cerebral palsy.

There are some possible culprits, says Ms
Khuu. For example, the muscles of individuals with cerebral
palsy have been found to have high levels of collagen, which
explains why the muscles are rigid. Levels of satellite
cells, the progenitor cells needed for muscle fibres to
regenerate when they have been damaged, are low.

The
problem is unlikely to be about one particular type of cell,
but a complex interaction of myriad cells. “We want to be
able to pinpoint those differences down to specific
mechanisms or pathways.”

She has identified a number of
different cell types which she will use as agents and, using
computational modelling, programme them to behave as they
would in a normal physiology. This will allow her to compare
that to the muscle environment of those with cerebral palsy.
A deeper understanding of the composition of the muscle
environment in those with cerebral palsy, at a molecular and
cellular level, could open up the potential for therapeutic
interventions.

Dr Julie Choisne, also from the ABI, will
talk about her project funded by the Health Research
Council, looking at bone deformation in children with
cerebral palsy.

Children with cerebral palsy typically
walk in a way that compensates for their abnormal muscle
activity, “in a way that is most efficient for them”.
However, it is not good for their bones,” says Dr Choisne.
“The mechanical load on the foot, for example, is
different from what it should be, which deforms the bones’
alignment in the ankle.”

Dr Choisne is drawing on the CT
scans of 200 children aged four to 18 years old, sourced
through the Victorian Institute of Forensic Medicine, to
build a paediatric population-based atlas of the normal
development of the tibia, femur and pelvis bones. She will
use that data to build a computational model of both normal
and abnormal development.

By combining that model with
wearable sensors developed at the ABI, she hopes this will
allow for a quick and inexpensive assessment of a child’s
gait, its potential impact on their musculoskeletal system,
and what interventions may help. If specialists could assess
a child as early as possible, teach and help them walk
better early on in their lives, “they might not even need
an assistive device to walk when they get older, and most of
them won’t end up in a wheelchair.”

Stephanie Khuu, Dr
Geoffrey Handsfield and Dr Julie Choisne will present their
research for a brighter future for children with cerebral
palsy, Monday, 16 SeptemberUniversity of Auckland,
Grafton Campus

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